Evans syndrome may be linked to other underlying autoimmune disorders

Evans syndrome may be a chronic state of immune system dysregulation, rather than a coincidental combination of immune cytopenias, and may be associated with other autoimmune or lymphoproliferative disorders and primary immunodeficiencies.

Because little data on Evans syndrome exist and most are pediatric, researchers retrospectively analyzed data from a 2005 survey performed throughout the Working Group on Thrombocytopenias of the European Association to better define the clinical spectrum and outcomes of Evan syndrome in adults. Data were analyzed from 68 patients.

The mean of age of patients was 56.4. At the time of diagnosis of autoimmune hemolytic anemia or immune thrombocytopenia, the mean age was 52; both diseases occurred simultaneously in 54.5% of cases. Autoimmune hemolytic anemia occurred first in 16% of patients, whereas immune thrombocytopenia occurred first in 29.5%.

At the time of diagnosis of Evans syndrome, 14.7% of patients had a concurrent autoimmune neutropenia. Evans syndrome was considered idiopathic in 50% of patients; the disease was linked to an underlying disorder in 34 cases.

All patients received at least one course of corticosteroids. Ninety-four percent were assigned to an initial dose ranging from 1 mg/kg to 2 mg/kg per day. An initial response rate was observed in 83%. Complete response was observed in half, partial response was observed in half and 17% were nonresponders.

In addition to corticosteroids, 73% of patients required at least one second-line therapy: 28% underwent splenectomy and 11 patients were assigned to rituximab (Rituxan, Genentech), according to the researchers.

The mean follow-up time was 4.8 years. At the time of analysis, 32 patients were in remission, 56% had complete or partial response during treatment and 12% had active disease while assigned to treatment. Twenty-four percent died.

“In clinical practice, true cases of Evans syndrome may well show or precede a variety of underlying diseases or conditions, which may influence both the management and outcome,” the researchers said.

The article is an extensive study of Evans syndrome in adults, which emphasizes its seriousness. The death rate is higher than for either autoimmune hemolytic anemia or immune thrombocytopenia by themselves, and the need for careful but aggressive therapy is apparent.

– Wendell Rosse, MD
HemOnc Today Editorial Board member

Michel M. Blood. 2009;114:3167-3172.